Cystine is an amino acid exchange of the productmethionine. We can distinguish two main reasons for increasing the concentration of cystine in the urine: the excessive accumulation of cystine in the kidney cells and malabsorption of cystine in the renal tubules. Cystine accumulation in cells is the result of a genetic defect. It is a violation of exchange called cystinosis. Intracellular and extracellular accumulation of cystine crystals detected not only in the kidney tubules and interstitium, but also in the liver, spleen, lymph nodes, bone marrow, peripheral blood cells, nerve and muscle tissue of other organs.
The manifestations of cystinuria
The disease manifests as inflammationvarious organs due to irritation cystine exchange their products (such as gastritis, inflammation of the duodenum ulcer, pyelonephritis, cystitis, biliary tract).
Treatment of cystinosis
If a child is suspected of cystinuria (found in the urine of the high content of cystine), and in his family there were cases of diseases urinary and biliary tract - you need a diet therapy.
Treatment of cystinosis and cystinuria includes diet, vysokozhidkostny treatment and drug therapy to urine alkalinization and increase the solubility of cystine.
The diet is based on restricting the administration with foodmethionine amino acid products, which is a precursor of cysteine. To do this, from the food are eliminated cottage cheese, fish, cheese, mushrooms, egg white. All other animal proteins are allowed to take food only in the morning, at lunch and in the evening we recommend a vegetarian table. This is due to the fact that cystine is accumulated in the body in the evening and night hours. Since methionine is necessary for the child's growing body growth, long-term use can not be a strict diet, so after 4 weeks from the start of diet therapy diet of the child expands and close to normal, but it is characterized by a strict exception of fish, cheese and eggs. The amount of liquid should be at least 2 liters / day is especially important to take fluid before bedtime. For alkalizing urine used citrate mixture, sodium bicarbonate, blemaren, alkaline mineral water.
In addition to diet, usedpharmacological agents - vitamins (pyridoxine, ascorbic acid), and sodium salts. During exacerbation assigned vegetarian (potato and vegetable) diet with total exclusion of animal proteins for 2-3 weeks.
Sample menu for the period of exacerbation
First breakfast: mashed potatoes with a salad of apples, carrots, cabbage, sour cream; coffee.
Second breakfast: boiled potatoes with onion, seasoned with vegetable oil; the infusion of rose hips.
Lunch: vegetable soup, vegetable stew, stewed fruit.
Snack: apples, marshmallows.
Dinner: salad, cake and jam, fruit juice.
Daily - white bread, butter.
Showing fruit juices, mineral water( "Smirnoff", "Slavyanovskaya", "Borjomi", "NAFTA") in the afternoon and at night for alkalizing urine, which prevents loss of cystine in the form of salts.
To improve the solubility and cystinepreventing crystallization appointed penicillamine. Since penicillamine inactivate pyridoxine, is concurrently assigned to vitamin B6 (pyridoxine), vitamin A and vitamin E. Antibiotic therapy is needed upon accession infection. In cystinosis successfully used a kidney transplant, which takes place before the development of the final stage of chronic renal failure. Kidney transplantation can significantly increase the lifespan of patients - up to 15-19 years, but the deposition of cystine crystals observed in the graft, which eventually leads to the defeat and the transplanted kidney.
In most cases, the corresponding mode,diet and drug therapy can achieve stable normalization of indicators in urine. In the absence of treatment or when the ineffectiveness of the most natural outcome dizmetabolicheskoy nephropathy is kidney stones and inflammation of the kidneys. The most common complication is the development of nephropathy dizmetabolicheskoy urinary tract infections, primarily pyelonephritis.